Experience with the oral iron chelator deferiprone in transfusion-dependent children.

OBJECTIVE: To establish efficacy and safety of deferiprone. DESIGN: Prospective study. SETTING: The Lady Ridgeway Hospital for Children, Colombo. PATIENTS: Transfusion-dependent children in the age group 1 to 15 years. INTERVENTION: Patients were given 75 mg/kg/day of deferiprone orally in divided doses. MEASUREMENTS: Efficacy of deferiprone therapy was assessed by 4 to 6 monthly serum ferritin (SF) assays. Safety of therapy was assessed by 4-weekly white cell counts and serum alanine aminotransferase (ALT) levels. The Z-score was used to assess the significance of the difference between the mean initial and final SF level. RESULTS: 82 patients received deferiprone therapy for a mean duration of 30 +/- 14 months. Initial SF levels ranged from 1115 to 12,165 micrograms/l with a mean of 5156 +/- 2631 micrograms/l. Final SF levels ranged from 312 to 15,285 micrograms/l with a mean of 2809 +/- 2380 micrograms/l (Z score 5.99; p < 0.001). Two (2.4%) children developed agranulocytosis which reverted to normal on discontinuation of treatment. 41 (50%) developed arthropathy and in 17 this was severe enough to require discontinuation of therapy. Serum ALT levels were raised in 35 (43%) patients but reverted to pretreatment values or lower despite continuation of deferiprone therapy. There was one death in a 9-year old child who developed diabetes mellitus and heart failure despite deferiprone therapy for 3 years. CONCLUSIONS: A final SF level < 2500 micrograms/l was achieved in 52% children. Severe arthropathy and agranulocytosis may necessitate permanent discontinuation of therapy.

A trial of deferiprone in transfusion-dependent iron overloaded children.

OBJECTIVE: To determine the efficacy and safety of deferiprone. DESIGN: Prospective study. SETTING: 5 paediatric medical units at the Lady Ridgeway Hospital for Children (LRHC), Colombo. PATIENTS: Transfusion-dependent iron overloaded children in the age group 2 to 15 years. INTERVENTION: Patients were given a total daily dose of 75 mg/kg of deferiprone orally in divided doses. MEASUREMENTS: Efficacy of deferiprone therapy was assessed by 4-monthly serum ferritin assays using the ELISA technique. Safety of deferiprone therapy was assessed by 4-weekly white cell counts, platelet counts and serum transaminase levels. The Z-test was used to assess the significance of the difference between the mean initial serum ferritin level and the mean subsequent serum ferritin level. RESULTS: 54 patients received deferiprone therapy for a mean duration of 9 +/- 3 months. Initial serum ferritin levels ranged from 1500 to 10,700 ng/ml with a mean of 5743. Subsequent serum ferritin levels, obtained in 48 patients ranged from 740 to 7300 ng/ml with a mean of 3558 (p < 0.001). In 47 of the 48 patients subsequent serum ferritin levels were lower than initial levels. One child developed severe neutropaenia, which reverted to normal on discontinuation of treatment. 11 children developed arthropathy, which responded to ibuprofen therapy combined in some cases with a reduction of the dose of deferiprone to 50 mg/kg/day. Serum transaminase levels were raised in 5 patients but reverted to pretreatment values or lower despite continuation of deferiprone therapy. CONCLUSIONS: Deferiprone is a safe and effective oral iron-chelating agent which can be used, under strict supervision, in transfusion-dependent iron overloaded children.

Dengue haemorrhagic fever in Sri Lanka.

This is a retrospective study of 354 suspected cases of dengue haemorrhagic fever (DHF), admitted to Lady Ridgeway Hospital (LRH), Colombo during 1996. The objective of the study was to determine the correlation between clinical, laboratory, radiological and serological diagnosis of DHF. Diagnosis was based on the haemagglutination inhibition test. Serologically, 177 cases had dengue infection and, of these, 143 children had DHF. There was only a 50% correlation between clinical suspicion of DHF and positive serology. There was 100% correlation between laboratory and radiological diagnosis of DHF and positive serology.

Plant poisoning: a hospital-based study in Sri Lanka.

This is a prospective hospital based study of 148 cases of plant poisoning seen by the author during a 12 year period. All cases were accidental. There were 2 deaths. Some measures to reduce the incidence of plant poisoning in Sri Lanka are listed.

Neonatal jaundice due to ABO incompatibility in Sri Lankan.

A prospective study was carried out on 101 neonates with jaundice due to ABO incompatibility. The direct Coomb's test was weakly positive in 4 cases. The indirect Coomb's test using the eluate was positive in 8 cases. In the maternal blood either IgG anti-A or anti-B haemolysin was present in high titre in every case. Phototherapy was given when the indirect serum bilirubin level exceeded 9 mg/dl. Exchange transfusion was done-in 39 cases, 9 babies requiring multiple exchanges. There were 2 deaths.

Perinatal deaths at the Castle Street Hospital for Women in 1993.

OBJECTIVE: To identify causes for perinatal mortality at the Castle Street Hospital for women. DESIGN: Retrospective analysis of clinical notes. SETTING: Castle Street Hospital for Women, Lady Ridgeway Hospital and Sri Jayawardanepura General Hospital. PATIENTS: All stillbirths and early neonatal deaths at the Castle Street Hospital for Women in 1993. RESULTS: There were 192 antepartum, 31 intra-partum and 120 early neonatal deaths. 264 deaths were in low birth weight babies. Pregnancy induced hypertension, cord round the neck, anencephaly, antepartum haemorrhage and idiopathic respiratory distress syndrome were the common causes of mortality. CONCLUSION: Most deaths may have been prevented with better obstetric and neonatal care.